Kawasaki disease is an acute self-limited vasculitis and is commonly seen in children. This case report is a rare complication of Kawasaki disease. A 5-year-old boy had persistent fever, associated with bilateral nonexudative conjunctivitis, cervical lymphadenopathy, and maculopapular erythema on his trunk. The patient was diagnosed as Kawasaki disease and was treated by intravenous immunoglobulins and aspirin. However, 3 days after recovery of the symptoms, he developed bilateral severe sensorineural hearing loss. Corticosteroid therapy of 40 mg/day was immediately administrated for 15 days; no improvement was noticed in the child's hearing as evidenced by serial audiometry follow-up.